17 year old with AIH

My 16 year old son was admitted to the hospital 02/02/15 after a trip to the emergency room. He was jaundice and had vomited a few times the weekend before. He was also fatigued a couple of weeks leading up to this, but I had brushed that off to a teenager sleeping a lot. His liver enzymes were AST 2616 and ALT 3070. So he wasn't going anywhere. After 9 days of testing viral, genetic (Wilson's etc), drug induced and autoimmune markers were all negative a transjugular biopsy was performed (INR was 1.7) but still no answer. The enzymes bounced around in the 2000's the whole time. Because he is under 18, he was transferred to Jackson Memorial in Miami (we live just south of Tampa). He was is acute liver failure.

After 3 mores weeks of tests there, and going through all the paperwork for him to be listed for a transplant, his labs began to improve. Still without answers he was sent home.

1 month later labs began to climb quickly and we had to take him back to Miami. We basically just sat there and waited for him to get worse. 2 weeks later his labs began to improve on their own, so he was sent home again.

During these stays he's had ultrasounds, CT scan, 3 liver biopsies, a bone marrow biopsy and been tested for everything there is a test for at least 3 times. Still without any answers.

On July 31st he had a 4th liver biopsy here in Tampa. His levels were far from normal- but not quite high enough for him to sit in the hospital. The biopsy results said : Stage 4/4 Fibrosis (Probable Cirrhosis).

In September the transplant team suggested trying Prednisone- because at that point "there is nothing to lose".

Well it worked. Within just a few weeks his AST was 35 and ALT was 84. The lowest they had been since this whole thing started. He is now on 75 mg of 6-MP and took his last dose of Predisone on Saturday (he started on 60 mg and has been weaned off).

He has an appointment in Miami tomorrow - a follow up with the transplant team. I am happy is doing better now- but I am worried about what is to come. I have a ton of questions. Can his liver repair itself at all at this point? His bilirubin is just slightly elevated but otherwise the liver function test is normal. Was he weaned off the Prednisone too fast? I have read others have been on it for 18 months and longer. He is a senior in high school and has had to give up things he loves like football- he can't get a job since we don't know what's going to happen next etc. Our lives are on hold for now until we get some answers. Does this mean he has AIH?

Post Edited (AutoHepMom) : 1/24/2016 7:38:40 AM (GMT-7)

Hi there

Looks to me like they think it is AIH but I would double check with them. Most people with AIH test positive for certain antibodies and that can go a long way to clinch the diagnosis. But some do not (it is called sero negative AIH) and I wonder if that is what happened with your son. The response to prednisone can then be the key to the diagnosis.

The speed at which doctors wean off pred varies. As you say some like to keep people on them for 12-18 months others get people off sooner. It causes a lot of side effects which is why they are keen to get people off them. I am not sure there is a definitive right or wrong answer on speed. If he is doing well on just the 6-MP then it looks OK.

As to the future - well it is frankly hard to say. I have had AIH for 16 years now and been on various online support forums. One person I know was diagnosed with AIH and cirrhosis at 16 and now some 25 years later she is still fine works full time in a busy busy job as a project manager and has 2 teenage daughters. I could also mention that a while back there was a guy playing international soccer who had AIH. But some others do suffer ongoing fatigue and find they do need to take things easy. I would say in every case that it takes a good few months to get over the initial illness. Treatment is long term often for life.

With cirrhosis caused by AIH there is a good chance the damage can be stopped in its tracks. You are monitored for complications of cirrhosis as they can be dealt with.

So in a nutshell I would ask the team to confirm or otherwise he has AIH and also ask for their views on the severity of the damage.

Ann

AutoHepMom i went looking and found this site ,it might help you understand why,what to do and what to expect . I hope this helps emedicine.medscape.com/article/172356-treatment

Hi, I am not sure if this will help you at all but I thought I should post anyways because I can relate to the situation you are in though mine is a little bit different. I have not been able to find anyone near by who is dealing with AIH especially near my age.

I am a 19 year old female and was recently diagnosed with Autoimmune Hepatitis Type II. My case started very similar to your sons. I had a case in the past of jaundice and it took me 8 months to fully come back to normal blood test results, though at the time we thought it was a gallbladder attack.

In November of 2015 I went to the emergency room because I was very tired, was starting to become jaundice was throwing up, etc. I was released with the flu and a ultrasound that showed "Fatty liver". Over the next week the doctors outside the hospital monitored my liver enzymes with blood tests every 2 days and on December 1 was admitted into the hospital with (INR of 2.7, AST 2464, ALT 3087, Bilirubin 421). I had every test as well done on me, in an attempt to find something that would come back positive to diagnose me was a transjugular biopsy, which came back positive for the Anti LKM-1 antibody (meaning I was type II AIH). The results showed over 60% of my liver dead and they brought in the transplant team because I was in acute liver failure. I was given 60 mg of prednisone once entering the hospital and was not given another dosage until 3 weeks later when I was tentatively released. I was on Vitamin K drips and NACs while in the hospital.

I am now at home with returning trips to a liver specialist who is trying to find the "Trigger" for my AIH. Plus frequent visits in and out of the hospital.
I have been on 35 mg of prednisone for 5 weeks, I have started tapering and I am now down to 30 with a 5 mg degrease every 2 weeks. Not going below 15 mg.
I am on 50 mg of Imuran, 1000mg Vitamin D, and Calcium. I have a 2 page list of side effects from the steroid which include drug induced type II diabetes ( on 2 insulins for that), kidney infections, internal bleeding, stomach ulcers, severe acne, skin/hair thinning, mood swings, nausea, headaches, adrenal glad deficiency (may or may not have to do with liver failure), my liver is now expanding and shrinking in a bad way which causes me a lot of pain.

Though I am only in the early stages of this tapering and condition my parents and I have done a lot of research and I have been seen by a lot of doctors. I know a good deal on the nutrition aspects, the condition itself, the drugs they have put me on, etc. Specifically tailoring it to liver failure and AIH. I have tried to understand this as much as possible even though it is such a hard thing to ever truly get a grasp on, and especially because I know it is really hard for my parents to watch me go through (though I have been told I am handling this well). I cannot fully relate to how your son is handling this, I mean when your in a position you could need a liver transplant, you will live with this forever and there is no guaranteeing it will never come back its not easy to cope. I am struggling with similar problems, I can't get a job because I don't know whats going to happen in the near or far future, I cannot go back to university this semester for the same reason, my friends treat me a bit different sometimes, but my friends and parents have been very supportive with doing things with me even if I do get tired easily.

It is a hard thing to be thrown into and defiantly a hard thing to cope with, as I have said many times to my parents you cannot take the irritation I get sometimes at the little things personally its just I went from being able to do all these things and now I cannot and I got handed this and now had to keep moving forward, at first all the unknown bothered me but patience is the key because this is one of those things that does not provide answers quickly.

I apologize for this being so long especially if it does not help you at all I just thought I would reach out for my own curiosity because as I said I have not found many if any people around me who have AIH and if I have they are nowhere near my age or close to what I went/am going through.

Thanks :)

Post Edited (MH5899) : 1/23/2016 7:24:46 PM (GMT-7)

To MH5589
I just re-read your post and noticed you mentioned stomach ulcers. The team in Miami warned us if these while on the steroid. They had my son take 40 mg of omeprazole (antacid medication) while he was on the Prednisone to prevent that. I just purchased these over the counter because the insurance didn't want to cover it for some reason.

MH5589 - His bilirubin is just slightly out of range. The direct bili at the lab we use is less than 0.2 is normal and two weeks ago his was 0.3. Other than that his liver & metabolic panels are within normal range. He goes for labs every other week now. He just had blood drawn on Thursday. I should have the results in Monday. Kind of nervous since this is the first test since he stopped the Prednisone. The AST & ALT have been in the normal range since Dec 1, 2015.

Even though his bilirubin is just barely outside of normal- I asked the transplant docs about it earlier this week. They said the bili is always last to go back to normal and they didn't seemed alarmed at all. Now that I think of it- I remember them telling me that when he was in the hospital too.

Interesting that they are looking for a link to the antibiotics. My son & husband ate at a Chinese buffet 3 weeks before Nick went into the hospital. They were both sick with vomiting and diarrhea for 2 or 3 days after that. My husband was more sick than my son. Not sure if that had anything to do with this- but we definitely won't be eating there again. I keep thinking maybe they picked up some virus that triggered this in Nick. But is it even possible for him to go into liver failure within 3 weeks? So many questions...

MH5589- Nick started the Prednisone in September at 60 mg per day and has been weaned off slowly. Well- what I think is slowly anyway. I have read that some people stay on it for up to 3 years. His team said "Predisone is a nasty drug so we want him to be off of it asap". I asked them on Tuesday if they thought he came off of it too fast? They don't think so- and added they can usually tell if there will be an issue at 10 mg. She said since he did fine at that dose & under she thinks he'll be fine with the 6-MP alone. I guess we see the results tomorrow.

He never had any jaundice or any health problems at all before this happened. The only thing I noticed was the fatigue. But I just thought- he's 16 he was probably up late on the phone or playing xbox. His urine had turned into the color of Pepsi - but I didn't find that out until I saw it after the first night in the hospital. I asked him why he didn't say anything. He said he thought he was just dehydrated.

He too had his wisdom teeth removed. But his were just done in October (2015). During the consultation with the oral surgeon we went over everything that had happened and gave him the list of meds he was on at that time: prednisone, he had just started the 6-MP, vitamin D, and the omeprazole. Because 2 of Nick's teeth had just broken through and the other 2 were not far behind, he said he would not need to put him to sleep and if Nick stayed on top of the salt water rinses- he wouldn't even need an antibiotic. I was a little nervous- but it went fine. The surgeon said he didn't want his liver to have to process anything else at the time.

I made one of those big binders too! When he was released from the hospital both times, they printed out all of the labs & results from the biopsies, scans & etc. That was VERY helpful. When they started talking about releasing him the first time I asked for a copy of the records. They told me where to go in the hospital to get them. When I got there they told me I had to have his birth certificate and that it would cost $1.00 per page. The doc in Tampa told us at 4 pm on a Tuesday that he would be going to Miami by Ambulance at 8 pm. I rushed home to pack for both of us- but I didn't think I would need his birth certificate. When I told the transplant team this they said that was crazy and had one of the med students print it for us.

Are you in the United States? I ask because you said your bili was in the 400's. That seems soooo high if the normal range is the same. I think the highest Nick's was was around 30 (direct).

Post Edited (AutoHepMom) : 1/24/2016 7:09:56 AM (GMT-7)

MH5589 - 6-MP is an immunosuppressive drug. I don't know for sure - but I want to say I read somewhere that the liver turns Imuran into what 6-MP is. Don't quote me on that though.

Nick had a little bit of nausea for a couple of days about a week after he started the 6-MP. But nothing since then - besides the suppressed immune system of course. We can see that on the cbc. But his numbers are not too far off from normal.

He gained 15 pounds or so when he started the Prednisone. It seemed like he was eating or looking for something to eat every time I walked by the kitchen. He still has some acne on his forehead but other than that the steroid symptoms are gone. He has always been a bigger kid (offensive lineman on the football team), so gaining that 15 or so pounds wasn't great for him. But he's really started to try and improve his diet & exercise and he has lost about 40 pounds in the last couple of months. Whenever he was not in the hospital- he was in school- except over the summer of course. He missed about 2 months total of school last year. This year he has only missed 6 or 7 days & 4 of those days were because of a trip. He was granted a wish from the Make-A-Wish foundation! I can send you a link to a video of it if you are interested in seeing it. It was so nice!!!! I cry every time I watch it!

He and my husband go to the gym at least 4 days a week. At the appointment on Tuesday the team said for him to do anything he wants (that we will allow of course). They said if wants to go to college out of the state- he should do that. That he should get a job if he wants to and just get back on track & move forward with all of the plans he had before this happened. She stressed it's important not to just sit around & wait for something to happen. He is responding well to the treatment. I think it will be good for his/my sanity too. He wants to get a tattoo in March when he turns 18 so we asked about that too. I don't want him too because I got a few when I was 16 & 17 and I wish hadn't now. His doctor didn't come right out and say no- but she just said he doesn't need to take another hit to his liver. She explained there is a chance he could get a viral hepatitus from it.

As far as we know- he doesn't have any other autoimmune diseases. Not yet anyway. I too have heard it's not uncommon to have more than one. The Miami team told us he should be tested once a year for thyroid issues, celiac disease, and rheumatoid arthritis. No adrenal gland issues as far as I know.

Are you trying to follow anything special as far as your diet goes? If so, can you tell if it's making a difference?

Post Edited (AutoHepMom) : 1/24/2016 3:39:15 PM (GMT-7)

It it is an immunosuppressant I would say you are correct, but its worth asking the doctors about up here just to see if there is a difference that could be better.

My mom was concerned when we started the prednisone about the increased appetite because we knew a few people who had the same thing where they just always needed to eat and gained a lot of weight, but they said once they were off the prednisone everything returned to normal. Wow that takes hard work and dedication to bring your weight down that much! My doctor said that the prednisone on its own makes you gain weight, I sww it in my face especially my cheeks. Oh wow so he still did go to school in between. That is wonderful to hear that he was granted a wish I would love to watch it, that foundation does such amazing things for so many people, I hope it made him feel a bit better dealing with this. I go to university out of province and was flown home mid semester to be seen in Toronto but I do want to return to normal life when this is all over so it is good to know your doctors are saying the same about just moving forward and living your life now even if you need to be more attuned to things your body does. Ah my mom's the same about my belly button piercing haha its hard with tattoos and piercings because there is always that risk.

Since I did have liver problems in the past I have always stuck to taking certain things out of my diet that my doctor said wouldn't benefit my liver such as:
- No fried food
- Tend to avoid shellfish because its high in salt
- Soy beverages
-Meats like bacon and ham
I always tried to do the warm water with lemon in the mornings, and always drank skim or almond milk and dairy products low in milk fat. I tended to get pain in my upper right abdomen area when I ate something that wasn't all that good for me or was hard on my liver so I always wrote down what food it was and tended to avoid it. It made a difference in how I felt energy wise because when the pains happened it did drain me a lot.

I lost about 25 pounds since being in the hospital but because I am a drug induced diabetic my diet is restricted a lot right now. I have had to play around with what affects my blood sugar the most while also sticking to things that are good for my liver so my diet may be a bit more restricted then necessary.
I eat about 1400 calories a day with the majority of it being vegetables and fruits like:
kale (high in vitamin K for your liver), spinach, blueberries
Meats: like pork, beef, steak, chicken also things high in protein like beans, nuts, hardboiled eggs, tuna, salmon.
Then I tend to do 45 min a day of exercise
I notice I am feeling better and have more energy and have a lot less pain in my liver, I have lost weight and I'm slowly gaining my muscle back where I lost it. I don't know if your son has tried the warm water and lemon in the mornings but I found it really helped.

The specialist specifically told me to stay away from soy products, herbal teas, anything like ginseng or herbal pills for cleanses or liver, do not do glutton free, shellfish, alcohol obviously, fried foods. He said to eat a high protein, calcium and fibre diet with low sugar, carbohydrate and fats. Did your doctors ever mentioned anything about cutting things out of or adding into your sons diet?

That is amazing what he got to do especially since he is such a football fan! Its inspiring to see foundations like that do things for people and especially inspiring to see how the people like your son benefit from them, after being through what he has he deserved a trip like that!

I always have tried when drinking water through the day to just throw some lemons in because I heard they help fight off infection, change up the flavour and are just good for you but then a few years ago when I first got hit with jaundice I did a sort of liver cleanse and read about how a lot of people drink a cup of warm lemon water in the morning because off all the things it does for you (aids digestion, helps your immune system, its a diuretic and flushes out the bad toxins, even helps with weight loss), but especially people with liver disease because of everything getting processed through your liver. I tried it for a few weeks and personally hate warm lemon water haha but it seemed to make me just feel better in general.

I don't blame him I love meats in general as well, I know they tell you to try to eat 5-7 small meals a day and that actually does help but its such a hard thing to do when you have a busy schedule going to school and things so good idea cutting meal sizes and adding snacks I need to start doing that.

I got warned my face would become "Moonish" but its slowly going back to normal, normally I would have 0 motivation when feeling like this to workout that much and with losing so much muscle laying in the hospital its tiring but it seems to work wonders for my diabetes glucose readings. Thats awesome he is back at school and doing well there! Slowly getting back into a regular routine and normal life again. I am determined to go back but my parents want me to be closer to home so that will be a discussion for a few months down the line haha Its a hard disease because there is not much anyone can do for you but the doctors and then the waiting and then more tests and then more waiting and its overwhelming the people who are there to support you through stuff like this, its amazing.